Goldenhar Syndrome

1SpecialPlaceReading CornerDisorders and DelaysSyndromesGoldenhar Syndrome

Goldenhar Syndrome is a craniofacial congenital condition which includes deformities of the face.It is also known as Oculoauricular Dysplasia or OAV. It usually affects one side of the face only. The chance of having another child with Goldenhar is less than 1% or less. A child with Goldenhar has about a 3% chance of passing it on to his or her children.

Features

  1. a partially formed or totally absent ear (microtia)
  2. the chin may be closer to the affected ear
  3. one corner of the mouth may be higher than the other
  4. benign growths of the eye
  5. a missing eye

The congenital deformities can cause the following problems in the affected child –

  1. hearing problems
  2. weakness in moving the side of the face that is smaller
  3.  dental problems
  4. the soft palate may move to the unaffected side of the face
  5. the tongue may be smaller on the affected side of the face
  6. fusion of the bones of the neck
  7. blindness

Cause

The cause of the syndrome is still uncertain. Multiple factors can be attributed, viz genetic and environmental both. Some problem with brachial arch development in the foetus during the 1st trimester of pregnancy has been suggested by researchers. Previously there has been an increased incidence in the children of Gulf War veterans.

Speech and Language characteristics

  1. Poor receptive language skills
  2. Poor expressive language skills
  3. Articulatory difficulties
  4. Voice and resonance problems ( especially when there is a cleft palate)
  5. Poor auditory skills of attention, identification, comprehension memory and sequencing.

Intervention

Depending on the severity of Goldenhar Syndrome, a child may have some or all of the following surgeries:

  1. lowering of the jaw on the affected side
  2. lengthening of the lower jaw
  3. 3 to 4 operations to rebuild the outer ear
  4. addition of bone to build up the cheeks
  5. soft tissue may need to be added to the face

Other than a craniofacial surgeon, other important team members are a speech language pathologist, an audiologist, an ENT specialist, an occupational therapist and a physical therapist.

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